Transitional Care for Pediatric Patients with Neuromuscular Diseases: A Health Technology Assessment

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Transitional Care for Pediatric Patients with Neuromuscular Diseases: A Health Technology Assessment

Jackie Tran, MD
University of Medicine and Dentistry of New Jersey, USA

HTAi 9th Annual Meeting, Bilbao
Integrated Care for a Patient Centered System
25 June, 2012

Published in: Health & Medicine
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Transitional Care for Pediatric Patients with Neuromuscular Diseases: A Health Technology Assessment

  1. 1. Transitional Care for PediatricPatients with Neuromuscular Diseases: A Health Technology Assessment Jackie Tran, MD University of Medicine and Dentistry of New Jersey, USA HTAi 9th Annual Meeting, Bilbao Integrated Care for a Patient Centered System 25 June, 2012
  2. 2. OutlineBackground Transitional Care Overview Neuromuscular Diseases OverviewObjectivesMethodsResults Barriers RecommendationsConclusions
  3. 3. BackgroundTransitional Care Overview
  4. 4. Unique Adult NeedsPhysical maturation Size Psychosocial maturation Endocrine management Identity Deformities Experimentation IndependenceSexual maturation Family/Friends Menstruation Fertility Intellectual maturation Pregnancy Education Vocation
  5. 5. Reality: Unmet Needs Increase hospitalization, particularly of ages 18-21© Pediatrics. 2011 Jul;128(1):5-13. Epub 2011 Jun 27.Adults with chronic health conditions originating in childhood: inpatient experience in childrens hospitals.
  6. 6. Reality: Unmet NeedsNon-adherence to medications over a lifetime inrenal transplant patientsDecrease clinic attendance and an average of 1.47health provider changes in diabetes type 1 patientsDecrease mobility in spina bifida patients duringtransitional yearsIncrease long-term morbidity and mortality in cysticfibrosis patients
  7. 7. BackgroundNeuromuscular Diseases Overview
  8. 8. Selected Neuromuscular Diseases of Childhood Congenitalmyopathies Acquired dystrophic (e.g., Duchenne) myotonic Guillain-Barré syndrome congenital myasthenia gravisspinal muscle atrophy polymyositiscongenital myasthenia toxic myopathyhereditary motor sensoryneuopathyFriedreich ataxia
  9. 9. EpidemiologyWorldwide prevalence of disabling, inheritedneuromuscular diseases: 1 in 3,500Increased survival rate to adulthood Some Survival Statistics in the Literature60% of males born with Duchenne between 1983 – 1987 survived past age 24 More than 33% of Charcot-Marie-Tooth Type 2 patients live past the third decade of life A patient with Type 2 SMA have survival rate of 68.5% at age 25
  10. 10. Unique CharacteristicsLow recognitionApparent functional deficitsInfamous progressive/regressive coursesMedia portrayalMulti-organ systems involvementLarge team investment
  11. 11. Equally Poor Transitional CareA glaring 60% of 850 people living with muscle disease rate transition from child to adult services as “poor” or “very poor” Muscular Dystrophy Campaign 2008 survey
  12. 12. Objectives & MethodsFinding Transitional Solutions
  13. 13. ObjectivesIdentify systematic and unique barriers to asuccessful transition of care for adolescentswith neuromuscular diseases (NMDs).Recommend comprehensive strategies toimprove the experience for patients, familymembers, and health providers alike.
  14. 14. MethodsSystematic literature review of publicationsbetween January 2000 and December 2011Informal survey of local clinicians’ opinions
  15. 15. ResultsOnly the beginning…
  16. 16. General ObservationsNotable publication increase on transitional careover the past 5 yearsEmphasis on the following diagnoses: diabetes, cancer, renal transplant, inflammatory bowel dz cerebral palsy and spina bifidaMost prolific countries: USA, Canada and UK Honorable mentions: Australia, France, Japan, Switzerland
  17. 17. All Transitional Care NMD-Relevant Publications Publications Number of 60+ 14Publications • generic: “chronic illnesses” & “special health care needs” & “congenital disabilities” & “developmental disabilities”Population • inter-mix with spina bifida &description • multiple diagnoses-specific cerebral palsy & “chronic publications neurological disorders” • Duchenne dystrophy
  18. 18. All Transitional Care NMD-Relevant Publications Publications • highlight need for transitional care • clinician & patient survey/commentariesPublication • anecdote / case reports Content • single-institution experience • review of medical topics on • general critiques of current transitional ages transition processes & models • subjective reports of wellness & satisfactionOutcome • individual programs: • health expenditure atmeasures transitional age as a secondary admissions & biomarkers (retrospective & limited to 1 year after transition) outcome
  19. 19. Models of Transitional CareDiagnosis-focused (most common) pediatric and adult provider collaboration Medical home-based (least common) primary care practitioner as coordinatorAdolescent-focused physical, physiological, social issues focused Transition-based outreach representatives from one facility
  20. 20. General BarriersAfterthought processProvider availability (absolute & time)Coordinator responsibilityDecision-making capacityAdvisors resourcefulnessInsurance coverageTransportation / ArchitecturalMedical recordsVentilator, dialysis
  21. 21. Emotional Barriers Patient & Family neglect abandonment Fear Anxiety Distrust Uncertainty Peds teamAdult team burden attachmentunfamiliarity over-confidence
  22. 22. Unique Barriers & Opportunities BarriersEven more limited provider availabilityInstitutionalized patientsAging care providersUncertain future Opportunities Assistive technology Malpractice insurance Insurance / resources for the very ill Association with spina bifida and/or cerebral palsy
  23. 23. RecommendationsEarly notificationIndividualized plan flexible but definitive date of transition assess cognitive/emotional and functional status gradual promote of independenceComprehensive approach patient’s and family members’ input interdisciplinary team
  24. 24. RecommendationsCoordinator designationPatient education self care and disease process healthcare system navigationLong-term planning vocation and education living situationResources availability
  25. 25. Advanced TopicsProgram sustainabilityProvider trainingTransition to palliative careSocioeconomic status effects
  26. 26. ConclusionsAll children with neuromuscular diseases needa transition plan before entering adulthoodSuccessful transition requires coordination ofservices across all providersEspecially for individuals affected by NMDs,the transition plan should incorporatepatient’s and family’s long-term goals
  27. 27. Primary ReferencesAbbott, D. Transition to adulthood for young men with Duchenne Muscular Dystrophy. International Journal of Integrated Care, 31December 2009 - ISSN 1568-415Centers for Disease Control and Prevention (CDC). Prevalence of Duchenne/Becker muscular dystrophy among males aged 5-24years - four states, 2007. MMWR Morb Mortal Wkly Rep. 2009 Oct 16;58(40):1119-22.Strehle EM. Long-term management of children with neuromuscular disorders. J Pediatr (Rio J). 2009 Sep-Oct;85(5):379-84.Tiffreau V, et al. Ann Readapt Med Phys. 2006 Dec;49(9):652-8. Epub 2006 Jun 27. [Transition in health care from youth toadulthood for disabled people].Ouyang L, et al. Health care utilization and expenditures for childrenand young adults with muscular dystrophy in a privately insuredpopulation. J Child Neurol. 2008 Aug;23(8):883-8. Epub 2008 Apr 10.Manzur AY, Muntoni F. Diagnosis and new treatments in musculardystrophies. J Neurol Neurosurg Psychiatry. 2009 Jul;80(7):706-14.Hill ME, Phillips MF. Service provision for adults with long-term disability: a review of services for adults with chronicneuromuscular conditions in the United Kingdom. Neuromuscul Disord. 2006 Feb;16(2):107-12. Epub 2006 Jan 19.Nomura Y. [Care continuity for patients with myasthenia gravis during transition from childhood to adulthood]. Nihon Rinsho. 2010Jan;68(1):39-44.Yoshioka M, et al. [Care continuity for patients with myopathy during transition of childhood to adulthood]. Nihon Rinsho. 2010Jan;68(1):53-6.Minicozzi A. Transition to adult care: another view. Pediatr Nurs. 2000 Jul-Aug;26(4):411-2.Katz JD, et al. Parents perception of self-advocacy of children with myositis: an anonymous online survey. Pediatr Rheumatol OnlineJ. 2011 Jun 7;9(1):10.Goodman DM, et al. Adults with chronic health conditions originating in childhood: inpatient experience in childrens hospitals.Pediatrics. 2011 Jul;128(1):5-13. Epub 2011 Jun 27.Rearick E. Enhancing success in transition service coordinators: use of transformational leadership. Prof Case Manag. 2007 Sep-Oct;12(5):283-7.Parker AE, et al. Analysis of an adult Duchenne muscular dystrophy population. QJM. 2005 Oct;98(10):729-36. Epub 2005 Aug 31.

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